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Hearing disorders
- | ICD9 = | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = D034381 | }} Hearing disorders (or hearing impairment or hearing loss) is a full or partial decrease in the ability to detect or understand sounds. Caused by a wide range of biological and environmental factors, loss of hearing can happen to any organism that perceives sound. Sound waves vary in amplitude and in frequency. Amplitude is the sound wave's peak pressure variation. Frequency is the number of cycles per second of a sinusoidal component of a sound wave. Loss of the ability to detect some frequencies, or to detect low-amplitude sounds that an organism naturally detects, is a hearing impairment. They include: *Autophony *Hyperacusis *Tinnitis *Tullio phenomena Loudness, frequency, and discrimination deficiencies Hearing sensitivity is indicated by the quietest sound that an individual can detect, called the hearing threshold. In the case of people and some animals, this threshold can be accurately measured by a behavioral audiogram. A record is made of the quietest sound that consistently prompts a response from the listener. The test is carried out for sounds of different frequencies. There are also electro-physiological tests that can be performed without requiring a behavioral response. Normal hearing thresholds are not the same for all frequencies in any species of animal. If different frequencies of sound are played at the same amplitude, some will be loud, and others quiet or even completely inaudible. Generally, if the gain or amplitude is increased, a sound is more likely to be perceived. Ordinarily, when animals use sound to communicate, hearing in that type of animal is most sensitive for the frequencies produced by calls, or, in the case of humans, speech. This tuning of hearing exists at many levels of the auditory system, all the way from the physical characteristics of the ear to the nerves and tracts that convey the nerve impulses of the auditory portion of the brain. A hearing impairment exists when an individual is not sensitive to the sounds normally heard by its kind. In human beings, the term hearing impairment is usually reserved for people who have relative insensitivity to sound in the speech frequencies. The severity of a hearing impairment is categorized according to how much louder a sound must be made over the usual levels before the listener can detect it. In profound deafness, even the loudest sounds that can be produced by the instrument used to measure hearing (audiometer) may not be detected. There is another aspect to hearing that involves the quality of a sound rather than amplitude. In people, that aspect is usually measured by tests of speech discrimination. Basically, these tests require that the sound is not only detected but understood. There are very rare types of hearing impairments which affect discrimination alone.eBook: Current Diagnosis & Treatment in Otolaryngology: Head & Neck Surgery, Lalwani, Anil K. (Ed.) Chapter 44: Audiologic Testing by Robert W. Sweetow, PhD, Jennifer McKee Bold, AuD, Access Medicine'' Types and causes of hearing impairment Hearing impairment comes from different biologic causes. Most commonly, the ear is the affected part of the body. Conductive Conductive hearing loss occurs when sound is not conducted properly through the outer ear, middle ear, or both. It is generally a mild to moderate impairment, because sound can still be detected by the inner ear. More severe impairments can occur, particularly in Otosclerosis. Generally, with pure conductive hearing loss, the quality of hearing (speech discrimination) is good, as long as the sound is amplified loud enough to be easily heard. Conductive hearing loss has a variety of causes: * Ear canal obstruction * Middle ear abnormalities: ** Tympanic membrane ** Ossicles * Inner ear abnormalities: ** Superior canal dehiscence syndrome * Other: ** Otosclerosis Sensorineural hearing loss A sensorineural hearing loss is due to insensitivity of the inner ear, the cochlea, or to impairment of function in the auditory nervous system. It can be mild, moderate, severe, or profound, to the point of total deafness. This is classified as a disability under the ADA and if unable to work is eligible for disability payments.http://www.eeoc.gov/facts/deafness.html Hearing impairment and the Americans with Disabilities Act The great majority of human sensorineural hearing loss is caused by abnormalities in the hair cells of the organ of Corti in the cochlea. There are also very unusual sensorineural hearing impairments that involve the VIIIth cranial nerve, the Vestibulocochlear nerve or the auditory portions of the brain. In the rarest of these sorts of hearing loss, only the auditory centers of the brain are affected. In this situation, central hearing loss, sounds may be heard at normal thresholds, but the quality of the sound perceived is so poor that speech can not be understood. Most sensory hearing loss is due to poor hair cell function. The hair cells may be abnormal at birth, or damaged during the lifetime of an individual. There are both external causes of damage, like noise trauma and infection, and intrinsic abnormalities, like deafness genes. Sensorineural hearing loss that results from abnormalities of the central auditory system in the brain is called Central Hearing Impairment. Since the auditory pathways cross back and forth on both sides of the brain, deafness from a central cause is unusual. Typical causes are discussed in following subsections. Long-term exposure to environmental noise Populations of people living near airports or freeways are exposed to levels of noise typically in the 65 to 75 dB(A) range. If lifestyles include significant outdoor or open window conditions, these exposures over time can degrade hearing. The U.S. EPA and various states have set noise standards to protect people from these adverse health risks. The EPA has identified the level of 70 dB(A) for 24 hour exposure as the level necessary to protect the public from hearing loss and other disruptive effects from noise, such as sleep disturbance, stress-related problems, learning detriment, etc. (EPA, 1974). Noise-Induced Hearing Loss (NIHL) typically is centered at 3000, 4000, or 6000 Hz. As noise damage progresses, damage starts affecting lower and higher frequencies. On an audiogram, the resulting configuration has a distinctive notch, sometimes referred to as a "noise notch." As aging and other effects contribute to higher frequency loss (6-8 kHz on an audiogram), this notch may be obscured and entirely disappear. Louder sounds cause damage in a shorter period of time. Estimation of a "safe" duration of exposure is possible using an exchange rate of 3 dB. As 3 dB represents a doubling of intensity of sound, duration of exposure must be cut in half to maintain the same energy dose. For example, the "safe" daily exposure amount at 85 dB A, known as an exposure action value, is 8 hours, while the "safe" exposure at 91 dB(A) is only 2 hours (National Institute for Occupational Safety and Health, 1998). Note that for some people, sound may be damaging at even lower levels than 85 dB A. Exposures to other ototoxins (such as pesticides, some medications including chemotherapy, solvents, etc.) can lead to greater susceptibility to noise damage, as well as causing their own damage. This is called a synergistic interaction. Some American health and safety agencies (such as OSHA and MSHA), use an exchange rate of 5 dB. While this exchange rate is simpler to use, it drastically underestimates the damage caused by very loud noise. For example, at 115 dB, a 3 dB exchange rate would limit exposure to about half a minute; the 5 dB exchange rate allows 15 minutes. While OSHA, MSHA, and FRA provide guidelines to limit noise exposure on the job, there is essentially no regulation or enforcement of sound output for recreational sources and environments, such as sports arenas, musical venues, bars, etc. This lack of regulation resulted from the defunding of ONAC, the EPA's Office of Noise Abatement and Control, in the early 1980s. ONAC was established in 1972 by the Noise Control Act and charged with working to assess and reduce environmental noise. Although the Office still exists, it has not been assigned new funding. Most people in the United States are unaware of the presence of environmental sound at damaging levels, or of the level at which sound becomes harmful. Common sources of damaging noise levels include car stereos, children's toys, transportation, crowds, lawn and maintenance equipment, power tools, gun use, and even hair dryers. Noise damage is cumulative; all sources of damage must be considered to assess risk. If one is exposed to loud sound (including music) at high levels or for extended durations (85 dB A or greater), then hearing impairment will occur. Sound levels increase with proximity; as the source is brought closer to the ear, the sound level increases. This is why music is more likely to cause damage at the same output when listened to through headphones, as the headphones are in closer proximity to the ear drum than a loudspeaker. With the invention of in-ear headphones, these dangers are increased. Genetic Hearing loss can be inherited. Both dominant genes and recessive genes exist which can cause mild to profound impairment. If a family has a dominant gene for deafness it will persist across generations because it will manifest itself in the offspring even if it is inherited from only one parent. If a family had genetic hearing impairment caused by a recessive gene it will not always be apparent as it will have to be passed onto offspring from both parents. Dominant and recessive hearing impairment can be syndromic or nonsyndromic. Recent gene mapping has identified dozens of nonsyndromic dominant (DFNA#) and recessive (DFNB#) forms of deafness. *The most common type of congenital hearing impairment in developed countries is DFNB1, also known as Connexin 26 deafness or GJB2-related deafness. *The most common dominant syndromic forms of hearing impairment include Stickler syndrome and Waardenburg syndrome. *The most common recessive syndromic forms of hearing impairment are Pendred syndrome, Large vestibular aqueduct syndrome and Usher syndrome. *The congenital defect microtia can cause full or partial deafness depending upon the severity of the deformity and whether or not certain parts of the inner or middle ear are affected. Disease or illness *Measles may result in auditory nerve damage *Meningitis may damage the auditory nerve or the cochlea *Autoimmune disease has only recently been recognized as a potential cause for cochlear damage. Although probably rare, it is possible for autoimmune processes to target the cochlea specifically, without symptoms affecting other organs.Wegener's granulomatosis is one of the autoimmune conditions that may precipitate hearing loss. *Mumps (Epidemic parotitis) may result in profound sensorineural hearing loss(90 Decibel|dB or more), unilateral (one ear) or bilateral (both ears). *Presbycusis is a progressive hearing impairment accompanying age, typically affecting sensitivity to higher frequencies (above about 2 kHz). *Adenoids that do not disappear by adolescence may continue to grow and may obstruct the Eustachian tube, causing conductive hearing impairment and nasal infections that can spread to the middle ear. *AIDS and AIDS-related complex|ARC patients frequently experience auditory system anomalies. *HIV (and subsequent opportunistic infections) may directly affect the cochlea and central auditory system. *Chlamydia may cause hearing loss in newborns to whom the disease has been passed at birth. *Fetal alcohol syndrome is reported to cause hearing loss in up to 64% of infants born to alcoholic mothers, from the ototoxic effect on the developing fetus plus malnutrition during pregnancy from the excess alcohol intake. *Premature birth results in sensorineural hearing loss approximately 5% of the time. *Syphilis is commonly transmitted from pregnant women to their fetuses, and about a third of the infected children will eventually become deaf. * Otosclerosis is a hardening of the stapes (or stirrup) in the middle ear and causes conductive hearing loss. * Superior canal dehiscence, a gap in the bone cover above the inner ear, can lead to low-frequency conductive hearing loss, autophony and vertigo Medications Some medications cause irreversible damage to the ear, and are limited in their use for this reason. The most important group is the aminoglycosides (main member gentamicin). Various other medications may reversibly affect hearing. This includes some diuretics, aspirin and NSAIDs, and macrolide antibiotics. Extremely heavy hydrocodone (Vicodin or Lorcet) abuse is known to cause hearing impairment. Commentators have speculated that radio talk show host Rush Limbaugh's hearing loss was at least in part caused by his admitted addiction to narcotic pain killers, in particular Vicodin and OxyContin. Physical trauma * There can be damage either to the ear itself or to the brain centers that process the aural information conveyed by the ears. * People who sustain head injury are especially vulnerable to hearing loss or tinnitus, either temporary or permanent. * Exposure to very loud noise (90 Decibel|dB or more, such as jet engines at close range) can cause progressive hearing loss. Exposure to a single event of extremely loud noise (such as explosions) can also cause temporary or permanent hearing loss. A typical source of acoustic trauma is an excessively loud music concert. Categories of hearing impairment Hearing loss is categorized by its severity and by the age of onset. Two persons with the same severity of hearing loss will experience it quite differently if it occurs early or late in life. Furthermore, a loss can occur on only one side (unilateral) or on both (bilateral). Types As discussed above, there are three major types of hearing loss: neural/sensorineural, conductive, or a combination of both. Treatment depends upon the type of hearing loss that is present. Quantification of hearing loss The severity of hearing loss is measured by the degree of loudness, as measured in decibels, a sound must attain before being detected by an individual. Hearing loss may be ranked as mild, moderate, severe or profound. It is quite common for someone to have more than one degree of hearing loss (i.e. mild sloping to severe). The following list shows the rankings and their corresponding decibel ranges: * Mild: ** for adults: between 25 and 40 dB ** for children: between 20 and 40 dB * Moderate: between 41 and 55 dB * Moderately severe: between 56 and 70 dB * Severe: between 71 and 90 dB * Profound: 90 dB or greater The quietest sound one can hear at different frequencies is plotted on an audiogram to reflect one's ability to hear at different frequencies. The range of normal human hearing (from the softest audible sound to the loudest comfortable sound) is so great that the audiogram must be plotted using a logarithmic scale. This large normal range, and the different amounts of hearing loss at different frequencies, make it virtually impossible to accurately describe the amount of hearing loss in simple terms such as percentages or the rankings above. Measuring hearing loss in terms of a percentage is debatable in terms of effectiveness, and has been compared to measuring weight in inches. Though in specific legal situations, where decibels of loss are converted via a recognized legal formula, one can infer a standardized "percentage of hearing loss" which is suitable for legal purposes only. Another method for determining hearing loss, is the Hearing in Noise Test (HINT). HINT technology was developed by the House Ear Institute, and is intended to measure an ability to understand speech in quiet and noisy environments. Unlike pure-tone tests, where only one ear is tested at a time, HINT evaluates hearing using both ears simultaneously (binaural), as binaural hearing is essential for communication in noisy environments, and for sound localization. Age of onset of hearing impairment The age at which the hearing impairment develops is crucial to spoken language acquisition. Post-lingual hearing impairments are far more common than pre-lingual impairments. If the hearing loss occurs at a young age, interference with the acquisition of spoken language and social skills may occur. Hearing aids, which amplify the incoming sound, may alleviate some of the problems caused by hearing impairment, but are often insufficient. Cochlear implants artificially stimulate the VIIIth Nerve by providing an electric impulse substituion for the firing of hair cells. Cochlear implants are not only expensive, but require sophisticated programming in conjunction with patient training for effectiveness. People who have hearing impairments, especially those who develop a hearing problem in childhood or old age, require support and technical adaptations as part of the rehabilitation process. Pre-lingual deafness Prelingual hearing impairment exists when the impairment is congenital or otherwise acquired before the individual has acquired speech and language, thus rendering the disadvantages more difficult to treat because the child is unable to access audible/spoken communication from the outset. It is important to note that those children born into signing families have no delay in language development and communication. Most pre-lingual hearing impairment is due to an acquired condition, usually either disease or trauma; therefore, families commonly have no prior knowledge of deafness. Post-lingual hearing impairment Post-lingual hearing impairment where hearing loss is adventitious after the acquisition of speech and language, usually after the age of six. It may develop due to disease, trauma, or as a side-effect of a medicine. Typically, hearing loss is gradual and often detected by family and friends of the people so affected long before the patients themselves will acknowledge the disability. Common treatments include hearing aids and learning lip reading. Loneliness and depression can arise as a result of isolation (from the inability to communicate with friends and loved ones) and difficulty in accepting their disability. Hard-of-hearing People who are hard of hearing have varying amounts of hearing loss but usually not enough to be considered deaf. Many people who are deaf consider spoken language their primary language and consider themselves "hard of hearing". How one classifies themselves relative to hearing loss or deafness is a very personal decision and reflects much more than just their ability to hear. The phrase hard of hearing, normally used as an adjective or adverb, can also be used as a noun, referring to people with hearing impairment as the hard of hearing. People who consider themselves culturally deaf, prefer the term "hard of hearing" or "deaf", and perceive "hearing impaired" as an insult. Hearing impaired persons with partial loss of hearing may find that the quality of their hearing varies from day to day, or from one situation to another or not at all. They may also, to a greater or lesser extent, depend on both hearing-aids and lip-reading. They may perhaps not always be aware of it, but they do admit to it being important to see the speaker's face in conversation. Many people with hearing loss have better hearing in the lower frequency ranges (low tones), and cannot hear as well or at all in the higher frequencies. Some people may merely find it difficult to differentiate between words that begin with consonantal sounds such as the fricatives or sibilants, z, or th, or the plosives d, t, b, or p. They may be unable to hear thin, high-pitched or metallic noises, such as birds chirping or singing, clocks ticking, etc. Often, they are able to hear and understand men's voices better than women's. Others will find their condition so much worse if circumstances in their immediate environment affect the way they are able to use their hearing aids, or prevent them from employing their speech reading skills. A room with a high ceiling and a lot of reverberation will affect the sound of a speaker's voice adversely. The position of the listener, too, sitting at a right angle to the speaker at a long seminar table, thus being able to hear only with one, maybe the ineffectual ear, can make a difference. Difficulties can also arise for the listener trying to lip-read, if the speaker is sitting with his back against the light-source and is in this way obscuring his face. A rule of thumb is that bright lighting is to the hearing-impaired what noise is to the hearing; a source of distraction. The speaker's accent; the topic under discussion, possibly with many unfamiliar words; the softness of his voice; possibly his having a speech impediment; a habit of holding a hand in front of his mouth or turning his face away at times: all these tendencies cause problems to the hard-of-hearing, especially when they have to rely on lip-reading. The rustling of papers, and notebook pages being turned are precisely the noises that will be the first thing hearing-aids pick up. Noisy situations are especially difficult, because hearing loss affects not only the ability to hear sounds, but also the ability to localize and filter out background noise. Unilateral hearing loss People with unilateral hearing loss (single sided deafness/SSD) can hear normally (or better) in one ear, but have trouble hearing out of the other ear. Problems with this type of deficit is inability to localize sounds (ie. unable to tell where traffic is coming from) and inability to process out background noise in a noisy environment, such as in a restaurant. Social impact Pre-lingual impairment See also: Prelingual deafness In children, hearing loss can lead to social isolation for several reasons. First, the child experiences delayed social development that is in large part tied to delayed language acquisition. It is also directly tied to their inability to pick up auditory social cues. This can result in a deaf person becoming generally irritable. A child who uses sign language, or identifies with the deaf sub-culture does not generally experience this isolation, particularly if he/she attends a school for the deaf, but may conversely experience isolation from his parents if they do not know sign language. A child who is exclusively or predominantly oral (using speech for communication) can experience social isolation from his or her hearing peers, particularly if no one takes the time to explicitly teach her social skills that other children acquire independently by virtue of having normal hearing. Finally, a child who has a severe impairment and uses some sign language may be rejected by his or her deaf peers, because of an understandable hesitation in abandoning the use of existent verbal and speech-reading skills. Some in the deaf community can view this as a rejection of their own culture and its mores, and therefore will reject the individual preemptively. Post-lingual impairment Those who lose their hearing later in life, such as in late adolescence or adulthood, face their own challenges. For example, they must adjust to living with the adaptations that make it possible for them to live independently. They may have to adapt to using hearing aids or a cochlear implant, develop speech-reading skills, and/or learn sign language. The affected person may need to use a TTY (teletype), interpreter, or relay service to communicate over the telephone. Loneliness and depression can arise as a result of isolation (from the inability to communicate with friends and loved ones) and difficulty in accepting their disability. The challenge is made greater by the need for those around them to adapt to the person's hearing loss. Many relationships have suffered because of the anger that occurs when there is general miscommunication between family members. Generally, it's not only the person with a hearing disability that feels isolated, but others around them who feel they are not being "heard" or paid attention to, especially when the hearing loss has been gradual. Many people opt not to choose hearing aids for fear of looking old, since hearing loss is usually associated with old age, which equals ineffectiveness in some societies. Family members then feel as if their hearing loss partner doesn't care about them enough to make changes to reduce their disability and make it easier to communicate. Medical treatments Approaches In addition to hearing aids there exist cochlear implants of increasing complexity and effectiveness. These are useful in treating the mild to profound hearing impairment when the onset follows the acquisitions of language and in some cases in children whose hearing loss came before language was acquired. Recent research shows variations in efficacy but some promising studies Elliot & Oliver'S Story - Researchshow that if implanted at a very young age, some profoundly impaired children can acquire effective hearing and speech.. Views of treatments There is controversy in the culturally deaf community as to whether cochlear implants address wellness concerns, the overall health and psycho-emotional well-being of prelingually deaf children at all. Gene therapy A 2005 study, achieved successful regrowth of cochlea cells in guinea pigs. It is important to note however, that the regrowth of cochlear hair cells does not imply the restoration of hearing sensitivity as the sensory cells may or may not make connections with neurons that carry the signals from hair cells to the brain. A 2008 study has shown that gene therapy targeting Atoh1 can cause hair cell growth and attract neuronal processes in embryonic mice. It is hoped that a similar treatment will one day ameliorate hearing loss in humans.PMID 18754012 Adaptations to hearing impairment Many hearing impaired individuals use certain assistive devices in their daily lives. Individuals can communicate by telephone using telecommunications devices for the deaf (TDD). This device looks like a typewriter or word processor and transmits typed text over the telephone. Other names in common use are textphone and minicom. A videophone can be used for distance communication using sign language. In 2004, mobile textphone devices came onto the market for the first time allowing simultaneous two way text communication. In the U.S., the UK, the Netherlands and many other western countries there are Telecommunications Relay Services so that a hearing impaired person can communicate over the phone with a hearing person via a human translator. Wireless, internet and mobile phone/SMS text messaging are beginning to take over the role of the TDD. Other assistive devices include those that use flashing lights to signal events such as a ringing telephone, a doorbell, or a fire alarm. Video conferencing is also a new technology that permits signed conversations as well as permitting an ASL-English interpreter to voice and sign conversations between a hearing impaired and hearing person, negating the need to use a TTY or computer keyboard. In addition, there are many new Telecommunications Relay Service technologies including IP Relay and captioned telephone. Resources Many different assistive technologies, such as hearing aids, are available to people who are hearing impaired. People with cochlear implants, hearing aids, or neither of these two devices also use additional communication devices to reduce the interference of background sounds, or to mediate the problems of distance from sound and poor sound quality caused by reverberation and poor acoustic materials of walls, floors and hard furniture. Three types of wireless, one-way wireless exist along with hard-wired devices. A wireless device used by people who use their residual hearing has two main components. One component sends the sound out to the listener, but is not directly connected to the listener with the hearing loss. The second component of the wireless system, the receiver, detects the sound and sends the sound to the ear of the person with the hearing loss. The three types of wireless devices are the FM system, the audio induction loop and the infra red system. Each system has advantages and benefits for particular uses. The FM system can easily operate in many environments with battery power. It is thus mobile and does not usually require a sound expert for it to work properly. The listener with the hearing loss carries a receiver and an earpiece. Another wireless system is the audio induction loop which permits the listener with hearing loss to be free of wearing a receiver provided that the listener has a hearing aid or cochlear implant processor with an accessory called a "telecoil". If the listener doesn't have a t-coil or telecoil, then she must carry a receiver with an earpiece. The third kind of wireless device for people with hearing loss is the infra red (IR) device which also requires a receiver to be worn by the listener. Usually the emitter for the IR device, that is, the component that sends out the signal, uses an AC adaptor. The advantage of the IR wireless system, is that people in adjoining rooms cannot listen in on conversations, and thus it is confidential and necessary for situations where privacy and confidentiality are required or chosen. Another way to achieve confidentiality is to use a hardwired amplifier which sends out no signal beyond the earpiece that is plugged directly into the amplifier. That amplifier of the hardwired device also has a microphone inside of it or plugged into it. * Hearing dogs, a category of assistance dogs, are trained to help those with hearing impairments. * The advent of the internet's World Wide Web and closed captioning has given the hearing impaired unprecedented access to information. Electronic mail and online chat have reduced the need for deaf and hard of hearing people to use a third-party Telecommunications Relay Service in order to communicate with the hearing and other hearing impaired people. See also * Audism, discrimination against Deaf and hard-of-hearing people * Auditory brainstem response (ABR) test * Cochlear implants * Conductive hearing loss * Deaf culture * Deaf history * Ear disorders * Hearing loss with craniofacial syndromes * King-Kopetzky syndrome * Models of deafness for a comparison of the medical, disability and cultural models of deafness. * Mondini Dysplasia * Noise induced hearing loss * Noise pollution * Post-lingual hearing impairment * Pre-lingual deafness * Tinnitus * Unilateral hearing loss References Bibliography U.S. Environmental Protection Agency press release, April 2, 1974 Quotations *"Blindness cuts you off from things; deafness cuts you off from people." — Helen Keller *"What matters deafness of the ear, when the mind hears. The one true deafness, the incurable deafness, is that of the mind." — Victor Hugo *"Deaf people can do anything except hear.". -- I. King Jordan External links *World Health Organization fact sheet on deafness and hearing impairment *National Association of the Deaf. The NAD protects deaf and hard of hearing civil rights. *International Federation of Hard Of Hearing Young People *Hard of Hearing Advocates Non-profit foundation dedicated to helping those with hearing loss Category:Audiology Category:Communication disorders Category:Hearing disorders Category:Otology